ISSN: 2578-465X
A Tale of Two Ectopics: Two Cases Depicting the End Spectra of Ectopia Lentis
Aim: To bring in perspective the two extremes of dysmorphic syndrome presenting with ectopia lentis.
Methods and Material: A nine year old female with history of bilateral gradual painless diminution of vision and a twelve year old female with similar complaints underwent a thorough systemic, ophthalmic and serological examination.
Results: Increased corneal diameters and bilateral supero-temporally displaced pupils with infero-nasally displaced lens were detected with normal systemic features and serology in the first patient who was subsequently diagnosed as a case of ectopia lentis et pupillae (ELeP). Temporally displaced lens with normally placed pupil was observed in the second patient who was subsequently diagnosed as a case of Marfan’s syndrome. Systemic evaluation revealed aortic root dilatation, mitral valve prolapse and arachnodactyly.
Conclusions: While the patient with ELeP had no systemic abnormalities and its management required only spectacle correction, Marfan’s syndrome patient had grave systemic abnormalities requiring a multi-speciality approach to its management. This paper highlights the two extremes of dysmorhic syndrome having ectopia lentis at presentation, with ELeP at one end of spectrum without any systemic involvement and Marfan’s syndrome at the grave end of the spectrum with plethora of dire systemic abnormalities.
Keywords:
Ectopia Lentis; Prolapsed; Arachnodactyly; Marfan’s Syndrome; Dysmorphic
